The Management of Desmoid Tumors: A Retrospective Study …Research Article The Management of Desmoid Tumors: A Retrospective Study of 30 Cases Yosr Zenzri ,1 Yosra Yahyaoui,1 Lamia

Research ArticleThe Management of Desmoid Tumors: A Retrospective Study of30 Cases

Yosr Zenzri ,1 Yosra Yahyaoui,1 Lamia Charfi,2 Zahra Ghodhbani,1 Feryel Letaief ,1

Mouna Ayadi,1 and Amel Mezlini1

1Medical Oncology Department, Salah Azaiez Institute, Tunis, Tunisia2Pathology Department, Salah Azaiez Institute, Tunis, Tunisia

Correspondence should be addressed to Yosr Zenzri; [email protected]

Received 16 April 2020; Accepted 1 July 2020; Published 18 July 2020

Academic Editor: C. H. Yip

Copyright © 2020 Yosr Zenzri et al. (is is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Objectives. Desmoid tumor also called aggressive fibromatosis is a rare type of benign tumor. It is a mesenchymal malignancywithout metastatic potential. (e standard management is resection, but other options including observation may bediscussed. Desmoid-type fibromatosis may occur throughout the body, but the abdominal wall is the most common site. (eaim of our study was to assess the clinicoepidemiological profile, prognostic factors, and treatment outcome of desmoidtumors. Methods. A monocentric retrospective study was conducted over a period of 19 years between February 2000 andNovember 2019 at the oncology department of Salah Azaız Institute. Our study concerns 30 patients with desmoid tumor.All data regarding patients were obtained from the medical record. Results. (irty patients were included. (e median agewas 35 years with a female predominance (sex ratio � 0.07). A palpable mass was the most common complaint (n � 27).Median tumor size was 5 cm.(e principal site of involvement was the abdominal wall (n � 14). Surgery was performed in 27patients. (e histopathology reports listed 14 (52%) cases with negative margins and 13 (48%) cases with positive margins.Radiation therapy was performed in 2 patients. One patient received tamoxifen. Local recurrence occurred in 11 patients.Two patients died of their desmoid tumor. Abdominal wall tumors have less risk of recurrence compared with other sites(p � 0.047). Macroscopic margin involvement (R2) was the only prognostic factor influencing disease-free-survival(p � 0.034). Conclusion. Desmoid tumors are aggressive tumors with a tendency for local recurrence. Abdominal walltumors have less risk of recurrence. Macroscopic margin involvement was the only prognostic factor that affects disease-free-survival.

1. Introduction

Aggressive fibromatosis also called desmoid tumors (DTs)are soft tissue malignancies originating from fascial planes,connective tissues, and musculoaponeurotic structures ofthe muscles [1]. DTs are rare and constitute less than 3% ofall soft tissue tumors and 0.03% of all neoplasms [2]. (eyare typically diagnosed in young adults with a female pre-dominance [3].

DTs are a benign proliferation of myofibroblasts. (isentity does not have the ability to metastasize. Althoughbenign, these tumors can be locally invasive, causing pain ordeformity that could make surgical removal more difficult.

(e clinical behavior and natural history of aggressivefibromatosis remain unpredictable.

(e symptoms depend on location and size of the tumor.(e presentation can vary from asymptomatic to severepain, swelling, deformity, and loss of function.

In fact, these tumors can be very aggressive with rapidgrowth and mass effect while others can be indolent andspontaneously regress [4].

(e exact etiology of these tumors is unknown buttrauma, hormonal, and genetic factors are considered to becorrelated with the development of DTs [5].

Wide surgical excision remains the mainstay for re-sectable aggressive fibromatosis. But, alternative treatment

HindawiInternational Journal of Surgical OncologyVolume 2020, Article ID 9197216, 7 pageshttps://doi.org/10.1155/2020/9197216

modalities including radiation therapy, chemotherapy, ta-moxifen, tyrosine kinase inhibitors, and nonsteroidal anti-inflammatory drugs may be used as a treatment for recurrentdisease or as primary therapy to avoid mutilating surgicalresection [6].

(e aim of our study was to assess the clinicoepidemio-logical profile, prognostic factors and treatment outcome ofDTs.

2. Materials and Methods

2.1. Study Design. A retrospective study including all con-secutive patients with DTs was performed at a tertiary-levelhospital (Salah Azaız Institute) from February 2000 toNovember 2019. (is study has been approved by the in-stitution’s Ethics Committee. Consent to participate waswaivered do to the retrospective nature of the study. Datawere collected and handled anonymously. (e diagnosis ofDTs was based on clinical, radiological, and histologicalcriteria. Only patients with histologically confirmed ag-gressive fibromatosis who received their treatment at a studycenter were included. (e histological diagnosis was madeby the pathologists of the institute.

2.2. Data Collection. Data extracted as part of this retro-spective analysis included age at diagnosis, gender, date ofdiagnosis, tumor size, primary site, surgical margins, re-currence rate, date of progression, time to progression, dateof last follow-up, and survival status.

Lesions at the buttock, axilla, and groin were classified asextremity. Lesions at the neck, because of their small numberin our study (n� 1), were considered with extremity lesions.

Margin was classified as gross positive (R2), microscopicpositive (R1), or negative (R0).

Recurrence was defined as a lesion that histologicalexamination has proven recurrent aggressive fibromatosis ora lesion that was deemed suspicious on imaging.

2.3. Statistical Analysis. (e data were analyzed on theStatistical Package for the Social Sciences (SPSS) version 20(IBM SPSS, Armonk, NY, USA). Results were expressed asmean± standard deviation. Univariate comparisons be-tween groups were performed using chi-squared tests fordichotomous variables or Fisher’s test when appropriate. Forcontinuous variables, independent samples t tests were used.P< 0.05 was considered to be significant.

Cumulative event rates were calculated using themethodof Kaplan–Meier. Survival curves were compared using thelog-rank test. Recurrence-free survival was determined asthe time from diagnosis to either histology-proven or ra-diologic evidence of disease recurrence.

3. Results

(e median age of the 30 patients at study inclusion was 35years (range, 18–80 years). Patients with aggressive fibro-matosis were predominantly female (n� 28; 93%). (e av-erage delay in consultation was 6 months.(ree patients had

history of a surgical procedure at the site of the tumor, while2 patients recalled nonsurgical trauma at the site of tumor.None of the patients had history of familial adenomatouspolyposis (FAP).

(e diagnosis of aggressive fibromatosis was madeduring the first trimester of pregnancy in one patient. Apalpable mass was the most common complaint (n� 27). Sixpatients were complaining of extreme pain. One patientpresent with a swelling and functional disability. DTs werelocated in the extremities (n� 9; 30%), abdominal cavity(n� 4; 13%), abdominal wall (n� 14; 47%), or thorax (n� 3;10%). (e median size of the tumor was 5 cm (range,2.5–17.5 cm).

Eighteen patients (60%) received magnetic resonanceimaging (MRI) examination, and nineteen (63%) had ul-trasonography. (e diagnosis was based on histologicalsamples obtained by core-needle biopsy in 20 patients(67%).Characteristics of the 30 patients included in ourstudy are detailed in https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3568525/table/T1/ Table 1.

Wide surgical resection was performed in 27 patients(90%). In 14 cases, a wide (R0) resection, in 11, a marginal(R1) resection, and in 2 cases, an intralesional (R2) re-section was achieved. Immunohistochemical analysisrevealed that the tumor cells were strongly positive forbeta-catenin in 10 patients (33%). Figures 1 and 2 illustratethe macroscopic and microscopic aspects of the desmoidtumors.

Two cases were managed by simple observation. Twopatients with R1 resection underwent adjuvant radiationtherapy. One patient with R2 resection received tamoxifenafter surgery.

One patient developed an acute respiratory failure anddied before starting any treatment.

Treatment characteristics are detailed in Table 2 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3568525/table/T1/).

(e median follow-up was 40 months. Local recurrence(LR) occurred in 11 (38%) patients within a median timeinterval of 21 months (range, 1–60 months). Of the 11patients with LR, 6 had positive margins after initial surgery.Eight patients (73%) underwent repeat resection, 2 patientswere lost to follow-up, and one patient developed bowelobstruction and died before resection. Two patients hadadjuvant radiation therapy. Four of these 8 patients had asecond relapse. An attempt at another resection was un-dertaken in three patients. One patient was lost to follow-up.One of these three patients developed a subsequent recur-rence during follow-up.

(e 1 and 3-year recurrence-free survival was 27 and18%, respectively (Figure 3).

Abdominal wall tumors had less risk of recurrencecompared with other sites (p � 0.047).

Macroscopic margin involvement (R2) was the onlyprognostic factor influencing disease-free-survival(p � 0.034) (Figure 4).

In univariate analysis, age, history of trauma, and size ofthe tumor did not show a significant impact on local re-currence. (e multivariate analysis could not be performeddue to the limited number of patients.

2 International Journal of Surgical Oncology

(a) (b)

Figure 1: Macroscopically, the tumors were tenacious, yellowish white on the cut.

(a) (b)

Figure 2: Interlacing bundles of fibroblasts separated by variable amounts of collagen in extra-abdominal fibromatosis. Regularly dis-tributed blood vessels are conspicuous.

Table 1: Characteristics of patients.

Treatment Frequency %SexMale 2 7Female 28 93Median Age� 35 yearsHistory of a surgical procedure at the site of the tumor 3 10History of nonsurgical trauma at the site of tumor 2 7History of pregnancy in females with tumor 1 3ComplaintsPalpable mass 27 90Pain 6 20Swelling and functional disability 1 3Location of primary tumorAbdominal wall 14 47Extremities 9 30Abdominal cavity 4 13(orax 3 10Median size of the tumor� 5 cmImaging ultrasonography 19 63MRI 18 60CT scan 12 40Biopsy 20 67

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4. Discussion

Aggressive fibromatosis is uncommon and accounts foronly 0.03% of neoplasms and less than 3% of soft tissuetumors [2].(ey are described as a clonal fibroblasticproliferation in soft tissues with infiltrative growth [7].(ey are locally aggressive tumors with a high rate of localrecurrence after surgery but rarely metastasize. Indeed,they have unpredictable clinical course. DTs can appear inany anatomic location, frequently in the abdominal region:in our study, the tumor was located in the abdominal wall(47%), abdominal cavity (13%), extremities (30%), andthorax (10%). Other studies suggested a completely dif-ferent distribution: abdominal wall (17.4%), abdominalcavity (10. 8%), and extra-abdominal (69.5%) [8]. (epresentation can vary from asymptomatic to pain, defor-mity, swelling, or loss of function. (e exact causes of DTsare largely unknown, but several factors may be involved.DTs usually occur in individuals between 15 and 60 yearswith a female preponderance [9]: 93% (n � 28) in our studyand 67% in other series [10]. But, there is an equal inci-dence in males and females in DTs associated with FAP[11]. Pregnancy is associated with DTs due to high es-trogenic levels [12]. DTs are related to trauma mainlysurgical ones: 16% of our patients had a history of trauma.It seems to be one of the most important etiologic factors.Indeed, this can be explained by molecular connectionbetween fibroproliferative disorders of mesenchymal tissueand wound healing processes [13].

DTs are mainly sporadic, but they can also be associatedwith familial infiltrative fibromatosis, hereditary desmoiddisease, Gardner syndrome, and familial adenomatouspolyposis .10 to 20% of DTs are associated with FAP while10–30% of patients with FAP develop desmoid tumors[14, 15]. Colonoscopy should be done for patients with DTsespecially if they are young or have intra-abdominal orabdominal wall tumor.

Histological examination of DTs revealed some char-acteristics: paucicellular proliferation of fibroblasts/myofi-broblasts in a dense collagenous background, spindle cellswith small and regular nuclei, pale eosinophilic cytoplasm,and acellular central area with increasing cellularity to theperiphery [16]. (e β-catenin positivity is caused byCTNNB1 mutation in chromosome 3p22. It is mainly foundin sporadic DTs [17]. In contrast, DTs associated with FAPare caused by germline APC mutation in chromosome 5(5q21e22) and then a somatic inactivation [18]. Nuclearpositivity of β-catenin supports the diagnosis of DTs.Moreover, some studies suggested that a very strongβ-catenin positivity is correlated with a high risk of recur-rence [19]. But, nuclear β-catenin is not always specific, itcan be positive in palmer/plantar fibromatosis, solitary fi-brous tumor, Gardner fibroma, synovial sarcoma, and low-grade fibromyxoid sarcoma and not all DTs will stain fornuclear β-catenin: cytoplasmic staining is more sensitive butless specific [20].

MRI is the modality of choice for assessment of thenature and size of DTs. 60% of our patients has undergonethis imaging. DTs show some features in MRI: they have

1.0

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.00 10.00 20.00 30.00 40.00 50.00 60.00Recurrence-free survival (months)

MarginsR2R0 ou R1

Recu

rren

ce-fr

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Figure 4: (e Kaplan–Meier plot of local recurrence according tomargin.

Table 2: Treatment characteristics.

Treatment Frequency %Wide surgical resection 27 90Surgical marginsR0 14 52R1 11 41R2 2 7Beta-catenin 10 33Observation 2 7Adjuvant radiation therapy 2 7Tamoxifen 1 3

Recu

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.00 10.00 20.00 30.00 40.00 50.00 60.00Recurrence-free survival (months)

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Figure 3: Recurrence-free survival analyzed by the Kaplan–Meiermethod.

4 International Journal of Surgical Oncology

stellar shape; they extend into the fascial planes and fat tissuein a sun-burst-like form. Besides, they are isointense on T1and hyperintense on T2 [21].

In most series, local recurrence rates after surgical re-section depend on margins. Indeed, these rates range from80% to 10% when surgical margins are positive or negative,respectively [22]. In our study, 11 patients (38%) had localrecurrence: 6 patients had PM and 5 patients had NM.

Macroscopic margin involvement (R2) was the onlyprognosis factor influencing the disease-free-survival(p � 0.034).

(erefore, positive margins seem to be the most im-portant indicator for an adjuvant treatment as radiationtherapy.

Many studies showed the importance of radiotherapy. Ina study published by Jelinek et al., adjuvant radiotherapy wasthe only significant prognostic factor for local control.(e 5-year local control rate was 53% for patients who underwentsurgery alone and 81% for patients who had surgery andradiation therapy (p � 0.018) [23].

(e effects of involved surgical margins after surgeryremain unclear. PM were predictors for failure in somestudies [24] but not in others [25].

In our study, abdomen wall tumors had less risk ofrecurrence compared with that in other sites (p � 0.047).(ere was no recurrence in a series of 7 abdominal walltumors published by Sutton and (omas [26]. Shao et al.reported a recurrence rate of 5.5% in a large series of 42abdominal wall tumors [27].

Other prognostic factors for recurrence have beenidentified, but they are still under investigation. In somestudies, tumors larger than 8 cm were more likely to recur(p � 0.021) [28]. Crago et al. discovered that tumors largerthan 10 cm were more likely to relapse [10]. Age at diagnosiswas a significant factor for recurrence in many studies.Crago et al. found more recurrence in patients younger than26 years [10]. In our series, tumor size and age were notfactors for local relapse.

(ere are multiple protocols to treat DTs: simple sur-veillance, surgery, chemotherapy radiation therapy, tyrosinekinase inhibitors, hormonal treatment, and nonsteroidalanti-inflammatory drugs.

Some series compared a simple surveillance protocolversus an active treatment and showed that the 5 yearsprogression-free survival is almost the same (49.9% versus58.6%) [29].

In other series, a spontaneous regression was observed in20% of cases [30].

(is result was in accordance with the wait-and-seemethod. However, when patients are symptomatic andexperiencing rapid or life-threatening progression and thetumor is resectable with negative margins, surgery seems tobe the best option [30]. But, mutilating surgical resectionand function loss are the disadvantages of surgery.

Systemic treatment options for aggressive fibromatosisthat are not amenable to surgery or radiation therapycomprise nonsteroidal anti-inflammatory drugs (NSAIDs),antihormonal therapies, tyrosine kinase inhibitors (TKIs),and chemotherapy.

Antihormonal therapies such as tamoxifen can be usedalone or in association with NSAIDs as first-line treatment.(ere are some suggesting that higher doses (up to 120mg/day) in combination with NSAIDs are more effective thantamoxifen alone [31].

COX-2 seems to play a role in the pathogenesis of DTs.NSAIDs has influence on the β-catenin pathway. NSAIDsthat inhibit COX may be an alternative treatment. Indo-methacin and sulindac were used in the treatment of DTs. Asa result, partial and complete response was achieved in somenonrandomized retrospective studies [32]. But, the risk ofcardiovascular events may be increased in patients receivingNSAIDs. (is treatment should be avoided for some fragilepatients.

(e chemotherapeutic option can be the treatment ofchoice in cases of unresectable, aggressively growing and/orlife-threatening tumors. Many chemotherapy protocols havebeen used. In the pediatric population, weekly adminis-tration of methotrexate and vinblastine has been evaluated.

Skapek et al. found that this regimen was well tolerated,and the response rate was 19% in a cohort of 28 patients [22].But, there is no prospective data for this combination inadults. Antracyclines appeared to have a higher responserate. (is regimen is administrated for 6 to 8 cycles [33].Pegylated liposomal doxorubicin at a dose of 50mg/m2 every4weeks showed acceptable toxicity and a significant activity[34]. Tyrosine kinase inhibitors (imatinib and sorafenib)have demonstrated activity in the treatment of DTs becauseof the PDGFRs expressed in tumor stroma.

In a series published by Gounder et al., Sorafenib wasadministered at 400mg oral daily. (ere was a 30% re-duction of the tumor size in 92% of the patients [35]. Penelet al. evaluated the efficacy of Imatinib in patients withunresectable and progressive symptomatic DTs. (is treat-ment showed that 83% of patients had stable disease; 3% hadcomplete response rate and 9% had partial response rate[36].

Due to the unpredictable behavior of DTs, such as longextended periods of stable disease and even rapid pro-gression, treatment must be individualized to improve theefficiency of local tumor control and protect the quality oflife. Consequently, the application of a multidisciplinaryassessment along with multimodality treatment shapes thefoundation of care for the related patients.

5. Conclusions

DTs are aggressive mesenchymal tumors with a tendency forlocal recurrence. Abdominal wall tumors have less risk ofrecurrence. Macroscopic margin involvement was the onlyprognostic factor that affects disease-free-survival. A mul-tidisciplinary approach is mandatory. Treatment recom-mendations including surgery, radiation therapy, andsystemic therapy are all evolving.

Data Availability

(e data that support the findings of this study are availablefrom the corresponding author upon reasonable request.

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Conflicts of Interest

(e authors declare no conflicts of interest.

Authors’ Contributions

Yosr Zenzri was involved in the concepts, design, literaturesearch, manuscript preparation, and statistical analysis;Yosra Yahyaoui was the guarantor and was responsible forconcepts, design, literature search, and manuscript prepa-ration; Lamia Charfi was involved in the definition of in-tellectual content and design; Zahra Ghodhbani wasresponsible for the literature search and manuscript prep-aration; Feryel Letaief and Mouna Ayadi prepared andreviewed the manuscript; Amel Mezlini was responsible forconcepts and definition of intellectual content.

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